Congenital heart disease - Treatment 

Treating congenital heart disease 

Congenital Heart Disease

Congenital heart disease refers to a series of birth defects that affect the heart. A paediatric cardiologist explains the different types of congenital heart disease and how each of them is treated.

Exercise

In the past, children with congenital heart disease were discouraged from exercising because it was felt that their health was too fragile to withstand the effects of vigorous physical activity. However, this is now thought to be inaccurate and exercise is believed to improve health, boost a child’s self-esteem and help prevent problems developing in later life.

There is no reason why most children with congenital heart disease cannot take part in normal physical activities, such as PE lessons, as long as:

  • their defect has been fully repaired with surgery
  • they do not have symptoms associated with congenital heart disease, such as shortness of breath during physical activity
  • their blood pressure is within the normal range
  • they do not have any complications of congenital heart disease, such as abnormal heartbeat

Even if your child doesn't satisfy these recommendations, they can still benefit from a more limited programme of physical activity, such as walking or, in some cases, swimming.

Your child’s heart specialist can give you more detailed advice about the level of physical activity that is suitable for your child.

Specific treatments for the most common types of congenital heart disease are described below.

Septal defects

Ventricular and atrial septal defects are treated in much the same way. The recommended treatment depends on the size of the defect and whether your child has any symptoms.

No treatment will be required if your child has a small septal defect that does not cause any symptoms. These types of septal defects have an excellent outcome and do not pose a serious threat to your child’s health.

If your child has a larger septal defect that causes symptoms such as extreme tiredness and failure to grow, a combination of medications is usually recommended. These may include:

  • diuretics – medication that removes fluid from the body, making breathing easier
  • angiotensin-converting enzyme (ACE) inhibitors – these widen the walls of the blood vessels, which should help reduce the strain on your child’s heart and improve their circulation
  • digoxin – medication that slows down the speed of your child’s heartbeat and increases the strength at which the heart pumps, which should help relieve symptoms

Your child may also be given high-calorie food supplements to help stimulate their physical growth.

If medication does not control your child’s symptoms, surgery will usually be required.

Two main types of surgery can be used:

  • open heart surgery – the surgeon makes a cut in the chest to access the heart and closes the septal defect, sealing it with a synthetic patch
  • transcatheter repair – a small tube called a catheter is guided to the heart through blood vessels, and special tools are passed down it and used to close the defect

Unlike open heart surgery, transcatheter repair does not cause any scarring and is associated with less pain after the operation and a quicker recovery.

However, transcatheter repair is a specialised technique that is only available at units that specialise in treating heart disease in children. Therefore, waiting times for this type of surgery may be longer than for open heart surgery.

Obstruction defects

Pulmonary stenosis

Mild pulmonary stenosis does not require treatment. This is because it does not cause any symptoms and is unlikely to pose a serious threat to your child’s health. However, in most cases, an annual check-up is likely to be recommended.

Moderate to severe cases of pulmonary stenosis usually require treatment, even if they cause few or no symptoms. Without treatment, pulmonary stenosis could lead to heart failure in later life. In these circumstances, a surgical technique known as a balloon pulmonary valvuloplasty is usually the preferred surgical option.

A balloon pulmonary valvuloplasty involves guiding a small tube (catheter) through the blood vessels to the site of the narrowed pulmonary artery. A balloon attached to the catheter is inflated, which helps to widen the pulmonary artery and relieve any blockage in blood flow.

Aortic stenosis

Treatment for aortic stenosis is usually delayed until your child develops symptoms, even if they are diagnosed shortly after birth. However, they will probably be referred for check-ups every six months.

Initial surgery for aortic stenosis is a balloon valvuloplasty, as described above. If this does not improve your child’s symptoms, it is usually necessary to remove and replace the valve in open heart surgery.

Replacement valves can be obtained from human donors, made from artificial materials, such as titanium, or taken from pigs before being modified for human use. Pigs are used because, biologically, their heart is closest to the human heart.

An alternative technique has recently become available that involves using a catheter to replace the aortic valve. The technique is known as a catheter insertion of a new aortic valve.

During the procedure, a catheter is passed through the groin and up into the heart. A replacement valve is then passed through the catheter and into position.

As catheter insertion of a new aortic valve is a new procedure, there is limited evidence about its long-term safety and effectiveness. This type of treatment is usually only recommended for people who are too ill or too weak to cope with the effects of open heart surgery.

Coarctation of the aorta

If your child has the more serious form of coarctation of the aorta that develops shortly after birth, they will be given medication, such as diuretics and digoxin, to help stabilise them. Surgery to restore the flow of blood through the aorta is usually recommended.

Several surgical techniques can be used, including:

  • removing the narrowed section of the aorta and reconnecting the two remaining ends
  • inserting a catheter into the aorta and widening it with a balloon or metal tube (stent)
  • removing sections of blood vessels from other parts of your child’s body and using them to create a bypass around the site of the blockage (this is similar to a coronary artery bypass graft which is used to treat heart disease) 

If your child develops the less serious form of coarctation of the aorta in later life, the main goal of treatment will be to control their high blood pressure. This will usually involve using a combination of diet, exercise and, in some cases, medication. Find out more about treatment for high blood pressure.

Cyanotic heart diseases

Tetralogy of Fallot (TOF)

Tetralogy of Fallot (TOF) is usually treated using surgery. If your baby is born with severe symptoms, surgery may be recommended soon after birth. If the symptoms are less severe, surgery will usually be carried out when your child is three to six months old.

Open heart surgery is usually used to treat TOF. During the procedure, the surgeon will seal the ventricular septal defect and widen the pulmonary valve.

Transposition of the great arteries (TGA)

Shortly after birth, your baby will be given an injection of medication called prostaglandin. This will prevent the ductus arteriosus closing after birth. The ductus arteriosus is the passage between the aortic and pulmonary valve.

Keeping the ductus arteriosus open means that oxygen-rich blood is able to mix with oxygen-poor blood, which should help relieve your baby’s symptoms. In some cases, it may also be necessary to use a catheter to create a temporary hole in the atrial septum (the wall that separates the two upper chambers of the heart) to further encourage the mixing of blood.

Once your baby’s health has stabilised, it is likely that surgery will be recommended. This should ideally be carried out during the first month of the baby’s life. A surgical technique called arterial switch is used. This involves detaching the arteries and reattaching them in the correct position.

Tricuspid atresia

As with TGA, if your baby has tricuspid atresia they will be given prostaglandin shortly after being born to encourage the mixing of oxygen-rich blood with oxygen-poor blood. The condition will then need to be treated using a three-stage procedure.

The first stage is usually performed during the first few days of life. An artificial passage, known as a shunt, is created between the heart and the lungs so that blood can enter the lungs. However, not all babies will need to have a shunt.

The second stage will be performed when your child is four to six months old. The surgeon will connect veins from the upper part of the body, which carry oxygen-poor blood, directly to your child’s pulmonary valve. This will allow blood to be pumped into the lungs, where it can be filled with oxygen.

The final stage is usually performed when your child is 18 to 36 months old. It involves connecting the remaining veins to the pulmonary valve. After this final stage, your child should be completely or mostly free of symptoms.

An effective alternative to this three-stage procedure would be to carry out a heart transplant, where a healthy heart is taken from a donor and surgically implanted into your baby. However, heart transplant surgery is rarely carried out in these circumstances because of a shortage of donated hearts that are a suitable size for a young baby.

Total anomalous pulmonary venous connection (TAPVC)

Total anomalous pulmonary venous connection (TAPVC) is also treated with surgery. During the procedure, the surgeon will disconnect the veins that are incorrectly connected to the right atrium, before reconnecting them to the left atrium.

The timing of surgery will usually depend on whether your child’s pulmonary vein (the vein that connects the lungs and heart) is also obstructed. If the pulmonary vein is obstructed, surgery will be performed after birth. If the vein is not obstructed, surgery can often be postponed until your child is a few weeks or months old.

Truncus arteriosus

Once a diagnosis of truncus arteriosus has been confirmed, it is likely that your baby will be given diuretics to help relieve their symptoms and stabilise their condition. Diuretics are a type of medication that removes fluid from the body.

Surgery will then be performed, usually within a few weeks after birth. The abnormal blood vessel will be split in two to create two new blood vessels, and each one will be reconnected in the correct position.

Patent ductus arteriosus (PDA)

Many cases of patent ductus arteriosus (PDA) can be treated with medication shortly after birth. Two types of medication have been shown to effectively stimulate the closure of the duct that is responsible for PDA. These are:

  • indomethacin
  • ibuprofen

If PDA does not respond to medication, a catheter can be used to seal the duct with a metal coil or plug.

Last reviewed: 25/07/2011

Next review due: 25/07/2013

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